Several mechanisms have been suggested for the development of hypoxemia in cirrhosis. A few patients of portal hypertension due to non cirrhotic liver disease with cyanosis have also been reported earlier. We report probably the first documented case of portal hypertension with portal cavernoma and a normal liver, who had intrapulmonary vascular dilatations leading to hypoxemia and cyanosis. Our case suggests that changes leading to hypoxemia can occur due to portal hypertension alone, in the presence of a normal liver.